Health-Related Quality of Life Long-Term Study in Lung Transplant Patients: A Single-Center Experience.

INTRODUCTION: Health-related quality of life (HRQL) assessment plays a crucial role in the follow-up care of lung transplanted (LTx) patients. Previous reports have indicated that the HRQL achieved by these patients is often poorer compared to that of healthy individuals. However, the factors contributing to this lower HRQL remain unclear. This prospective study aimed to assess the effectiveness of using both a generic and a disease-specific HRQL instrument in evaluating the outcomes of patients who have undergone LTx. METHODS: A total of 111 LTx patients were enrolled in the study, with 88 survivors completing the 5-year follow-up and 23 nonsurvivors identified within the first 3 y. Among the participants, 84 underwent double LTx, while 27 received a single LTx. Patients were interviewed before LTx, at 6 mo post-transplantation, and annually thereafter. Two validated instruments were utilized: the Euro quality of life five dimensions, a generic measure, and the St. George's Respiratory Questionnaire (SGRQ), a disease-specific questionnaire. RESULTS: The study showed significant improvements in Euro Quality of Life five Dimensions scores from 6 mo after LTx. Specifically, the percentage of patients without Mobility problems increased from 23% before LTx to 71% at 5 y (P = <0.001), while the ability to self-care improved from 48% to 100% (P = <0.001). The ability to carry out usual activities improved from 13% to 86% (P = <0.001), and the proportion of patients without anxiety and depression increased from 50% to 86% (P > 0.004). However, there was no significant improvement observed in Pain, with only a slight reduction from 57% to 42.8% (P = 0.22). The SGRQ also showed improvements in all dimensions (symptoms, impact, activities) (P < 0.001). However, by the fifth year, the HRQL scores remained below normal reference values. Chronic graft dysfunction was associated with a decline in SGRQ scores. Bilateral LTx patients exhibited better SGRQ scores compared to unilateral LTx patients from the first year post-transplantation. Notably, there were no differences in scores between nonsurvivors and survivors. CONCLUSIONS: The study highlights the long-term improvement in HRQL among LTx patients, with greater improvements observed in physical dimensions compared to psychological dimensions. Bilateral LTx was associated with better SGRQ scores than unilateral LTx, and chronic graft dysfunction primarily affected SGRQ scores. These findings underscore the importance of utilizing both generic and specific HRQL instruments in assessing LTx outcomes.

Hypoparathyroidism and medium-chain Acyl-CoA dehydrogenase deficiency, an unusual association.

OBJECTIVES: Hypoparathyroidism (HypoPT) is a rare disorder and non-surgical cases require careful evaluation, since may be due to genetic, autoimmune, or metabolic factors. CASE PRESENTATION: We present a 15-year-old girl with a previous diagnosis of medium-chain acyl-CoA dehydrogenase (MCAD) deficiency due to G985A homozygous mutation. She was admitted to the emergency department with severe hypocalcaemia and inappropriately normal level of intact parathyroid hormone. Main etiologies of primary HypoPT were excluded, so it was suspected to be related to MCAD deficiency. CONCLUSIONS: The association of fatty acid oxidation disorders and HypoPT has been previously described in the literature, but its link to MCAD deficiency has only been reported once. We present the second case describing the coexistence of both rare diseases. Since HypoPT can be a life-threatening condition, we suggest calcium levels be assessed in these patients on a regular basis. Further research is needed to better understand this complex association.

The next step after anti-osteoporotic drug discontinuation: an up-to-date review of sequential treatment.

Several antiresorptive drugs, like bisphosphonates and denosumab, are currently available for the treatment of osteoporosis due to their evidenced efficacy in reducing fracture risk at mid-term. Osteoanabolic therapies, like teriparatide, whose treatment duration is limited to 2 years, have also shown efficacy in the reduction of fracture risk. However, depending on the severity of osteoporosis and the presence of other associated risk factors for fracture, some patients may require long-term treatment to preserve optimal bone strength and minimize bone fracture risk. Given the limited duration of some treatments, the fact that most of the antiresorptive drugs have not been assessed beyond 10 years, and the known long-term safety issues of these drugs, including atypical femoral fractures or osteonecrosis of the jaw, the long-term management of these patients may require an approach based on drug discontinuation and/or switching. In this regard, interest in sequential osteoporosis therapy, wherein drugs are initiated and discontinued over time, has grown in recent years, although the establishment of an optimal and individualized order of therapies remains controversial. This review reports the currently available clinical evidence on the discontinuation effects of different anti-osteoporotic drugs, as well as the clinical outcomes of the different sequential treatment regimens. The objective of this article is to present up-to-date practical knowledge on this area in order to provide guidance to the clinicians involved in the management of patients with osteoporosis.

Trabecular Bone Score, Bone Mineral Density and Bone Markers in Patients with Primary Hyperparathyroidism 2 Years After Parathyroidectomy.

Following a parathyroidectomy there is a bone mineral density (BMD) improvement in patients with primary hyperparathyroidism. However, data of bone microarchitecture are scarce. Trabecular bone score (TBS) estimates bone microarchitecture and could provide valuable information in those patients. The aim of this study is to assess TBS changes 2 years after successful surgery in a group of patients with primary hyperparathyroidism and correlate these results with changes in BMD and bone turnover markers. This is a prospective study including 32 patients. In all participants BMD and TBS were measured, before and 24 months after surgery. Biochemical data: serum calcium, PTH, 25-OH-vitamin D, beta-crosslaps, bone alkaline phosphatase, and osteocalcin. 25 female and 7 male patients, mean age 64.6±12.4 years, were included in the study. At baseline, BMD was low at: lumbar spine (T-score -2.19±1.31), total hip (-1.33±1.12), femoral neck (-1.75±0.84), and distal one-third radius (-2.74±1.68). Baseline TBS showed partially degraded microarchitecture (1.180±0.130). After parathyroidectomy lumbar spine BMD increased significantly (5.3±13.0%, p<0.05), as well as total hip (3.8±8.8%, p<0.05). There was an increase in TBS, but this was not significant. There was a correlation between TBS and BAP at baseline (rs=0.73; p<0.01) and TBS and BAP 2 years after surgery (rs=0.57, p<0.05). Although bone density improves 2 years after surgery in patients with primary hyperparathyroidism and there is a restoration of bone turnover markers, TBS is not completely restored. These results remark the necessity of longer periods of study, to confirm if bone microarchitecture could be completely restored after surgery.

Trabecular bone score and bone mineral density in patients with postsurgical hypoparathyroidism after total thyroidectomy for differentiated thyroid carcinoma.

BACKGROUND: Although bone mineral density is reported to be increased in patients with postsurgical hypoparathyroidism (postsurgical HypoPT), the effect of HypoPT on trabecular bone score remains unknown. This study evaluated the long-term effects of HypoPT secondary to total thyroidectomy for differentiated thyroid cancer on trabecular bone score, bone mineral density, and bone turnover markers with a similar group of patients without HypoPT. METHODS: Women with resected differentiated thyroid cancer and either postsurgical HypoPT (n = 25; 8 premenopausal and 17 postmenopausal) or euparathyroid function (n = 98; 14 premenopausal and 84 postmenopausal) were matched for age and body mass index. Patients received thyroid-stimulating hormone suppression during follow-up. The bone mineral density and trabecular bone score were analyzed using dual x-ray densitometry and Med-Imaps software at baseline (1-3 months postsurgery) and at the final study visit. RESULTS: Follow-up duration was similar in studied groups (median 10 years). Baseline bone mineral density and trabecular bone score were similar between HypoPT and non-HypoPT patients, regardless of menopausal status. At study end, postmenopausal HypoPT patients had greater bone mineral density versus the non-HypoPT patients at the lumbar spine, hip, and distal radius (P = .001), and a greater trabecular bone score (1.31 ± 0.09 vs 1.24 ± 0.12, P = .0184). Premenopausal patients with and without HypoPT had similar bone mineral density values at the final evaluation. The bone turnover markers (osteocalcin, bone-specific alkaline phosphatase, and ß-crosslaps) were less in postmenopausal HypoPT patients, reflecting decreased bone turnover. CONCLUSION: Postmenopausal patients who underwent a total thyroidectomy for differentiated thyroid cancer with postsurgical HypoPT have greater trabecular bone score and bone mineral density compared with euparathyroid patients, suggesting that HypoPT protects against the negative effects of long-term thyroid-stimulating hormone suppression treatment on bone.

Quantitative ultrasound of the calcaneus in long-term liver or cardiac transplantation patients.

Bone loss is one of the most common complications after solid-organ transplantation, but it is frequently under-diagnosed. Our purpose was to evaluate quantitative ultrasound of calcaneus (QUS) in comparison with dual-energy X-ray absorptiometry (DXA) to identify transplant recipients with osteoporosis. We have cross-sectionally evaluated 140 transplant recipients (85 liver and 55 cardiac transplantations; mean age: 53.6 years, time since transplantation: 67.9 months). Devices used were Hologic 4500 QDR for DXA measurements and Sahara Clinical Sonometer (Hologic Inc, Bedford, MA) for calcaneal QUS. Quantitative ultrasound index (QUI) was calculated from speed of sound (m/s) and broadband ultrasonic attenuation (dB/MHz). QUI T-score and bone mineral density (BMD) T-score (spine and hip) were obtained from Spanish normative data. According to World Health Organization criteria, defined either at lumbar spine or femoral neck, 61% of the females had osteopenia and 32% had osteoporosis, whereas 52% of the males had osteopenia and 11% had osteoporosis. Calcaneal QUS parameters (speed of sound, broadband ultrasonic attenuation, and QUI) were positively correlated with lumbar and femoral BMD (p<0.001). In receiver operator characteristic analysis, a T-score QUI